(Data were presented as No. (%), the measurement data were described as
the mean ± standard deviation, the count data as medians and quartiles.
Abbreviations: GA, gestational age; IQR, interquartile range; SD,
standard deviation; PH, pulmonary hypertension; HFOV, high-frequency
oscillator ventilation;ECMO, extracorporeal membrane oxygenation.)
All cases had evidence of
pulmonary hemorrhage and hyaline membrane formation with varying
degrees, as well as alveolar septa, interstitial telangiectasia
congestion. Abnormalities of lobation of the lung and pulmonary
dysplasia were observed in three cases; Cardiac findings included PDA,
PFO, increased right ventricular volume, and mild congestion and
swelling of myocardial fibers microscopically; the liver herniated into
the thoracic cavity was found significant impression with normal hepatic
lobular structure with hepatocyte swelling and degeneration, and
significant congestion microscopically; diffuse congestion and bleeding
in other organs were observed. The diaphragmatic muscle tissue on the
defect side was significantly reduced, hyperplastic fibrous connective
tissue was more common, and the contralateral side was normal. The
autopsy results showed a consistence with the clinical cause of death.
Macroscopically, the lung volume of the ipsilateral side was
significantly smaller than the contralateral side (Fig 1 A), the average
proportional weight of ipsilateral vs. total lung weight (%) was
25.06%±6.99%, the average LBWR
was 0.0030±0.0023. The surface of the lung tissues was dark red and
smooth. And the floating test in water was positive. 3 (37.5%) of them
had lobulated deformity of the ipsilateral lung while bilobate,
trilobate and quadrilobate lungs were found; We found 4 patients with
right CDH had poor air content in both lungs. And 1 patient with left
large defect had severe right curvature of the spine, involving the T5-
L5 interval, showing that the left thoracic cavity was significantly
enlarged compared with the right thoracic cavity.
Microscopically, diffuse
pulmonary hemorrhage and hemosiderin laden macrophages were observed in
bilateral lung; hyaline membrane was formed in 7 cases; diffuse acute
and chronic inflammatory cell infiltration was observed in alveolar
space, alveolar septum was significantly widened, and capillaries were
dilated and congested; exfoliated mucosal epithelial cells, inflammatory
exudates and hyaline membrane were observed in small airways, which
completely blocked the lumen (Fig 1 B-E); interstitial vessels were
significantly dilated and congested with significant edema, pulmonary
dysplasia was only found on the ipsilateral sides in 2 cases,
bilaterally poor development was observed in 2 cases (bilateral
congenital eventration and right CDH)(Fig 1 D-E), acinar hypoplasia was
found in the pseudoglandular and canalicular stages of lung development
with reduced alveoli and small lumen. 4 patients (50%) had fair lung
development (1 on the left side and 3 on the right side) (Fig 1 B-C).
Muscular arteries showed media thickening, sometimes with complete
occlusion of the lumen (Fig 2).